Pathology
Most common hemoglobinopathies
- RBCs do not carry the normal Hgb but instead carry a less effective type |
Severe chronic blood disorder that occurs 1/2,000 per year in U.S |
Glutamic acid is replaced w/ valine in Hgb molecule → elongated RBC that is rigid & sickle w/ a shortened life spa |
Significant anemia may occur when RBCs sickle |
When cells sickle, blood becomes more viscous b/c cells clump together & prevent normal blood flow to the tissues of that area since their shape cannot pass through the smaller capillaries & venules → vaso-occlusive process leads to local tissue hypoxia → ischemia → infarction & pain crisis |
Hemolysis occurs following sickling |
Etiology / Risk Factors
Inherited autosomal recessive pattern |
African (1 in 400, 8% carry trait), Mediterranean, Middle Eastern, & Indian descent |
Passed on when both parents have gene or trait |
May be triggered by stress or traumatic event |
25% risk of Hgb SS, 25% Hgb AA, 50% Hgb AS |
Infection, fever, acidosis, dehydration, physical exertion, excessive cold exposure, hypoxia |
Hgb AS = carrier & usually have only minimal health problems |
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Complications
Cardiomegaly, fxn murmur |
Pulmonary HTN, Restrictive lung disease |
Retinopathy |
Cholelithiasis & gallstones |
Jaundice, hepatomegaly |
Functional Asplenia |
Chronic leg ulcers |
MODs common in adulthood |
Stroke |
Sepsis |
Delayed G&D & puberty |
Organs |
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Signs & Symptoms
Infants asymptomatic until 3-4 months d/t Hgb F protection (later half of 1st year of life) |
Pain crisis, recurrent pain episodes (vaso-occlusive)
- ↑ tachycardia & tachypnea → more sickling
- Most common in joints (hot, swollen) |
Acute Chest Syndrome (ACS)
- Cell clumping in lungs
- ↓ gas exchange → hypoxia, wheezing, cough, chest pain, fever → more sickling |
Aplastic crisis (profound anemia) |
Dactylitis (hand-foot syndrome), aseptic infarction |
Pale mucous membranes |
Easily fatigued w/ poor appetite |
↓ BP d/t severe anemia or ↑ BP d/t SC nephropathy |
Acute abdominal pain (most common) d/t sludging & splenomegaly |
Sequestration crisis / Splenic sequestration
- Pooling of blood in liver & spleen w/ ↓ blood volume & shock |
Bacterial meningitis or sepsis |
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Bone infarction |
Nursing Interventions
Immunizations & ABX to ↓ risk of infection |
Tx underlying cause (infection) |
H-O-P to it! → Hydration, O2, Pain relief |
O2 during episodes of crisis to prevent further sickling |
↑ fluids to promote hemodilution, 150 mL/kg/day or double maintenance w/ hypotonic, D5W or D5 w/ 0.25% NS |
Adequate pain management helps ↓ stress; always believe pain level |
NO PRN pain meds, use fix dose, can use w/ non-pharmacologic techniques |
Assess pain w/ the right pain tool & look for complications of pain |
Assess for S/S of ineffective tissue perfusion |
Avoid sudden temp change (cooling mattress for fever) |
Cluster care |
Quiet environment & privacy |
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Diagnostics
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Sickle-Turbidity Test (Sickledex) finger stick Possibility or SCA or SC trait
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Hgb Electrophoresis Dx, only accurate test for SCA
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Hgb ~6-9 mg/dL (normal 11-15 in infant)
Significantly lower w/ splenic sequestration, ACS, or aplastic crisis
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Reticulocyte Count ↑ greatly
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Peripheral Blood Smear Presence of sickle-shaped cells & target cells
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Platelet Count, Erythrocyte Sedimentation Rate, ↑
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LFTs ↑ bilirubin
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X-Ray Studies or Scans Determine extent of organ or tissue damage d/t vaso-occlusion
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K+ ↑ d/t hemolysis of RBCs after transfusion
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Pulmonary Infiltrate W/ ACS
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Collaborative Care & Meds
Stem cell transplant |
Splenectomy |
Prophylactic ABX |
Immunizations |
Analgesics |
O2 |
IV fluids |
PRBCs |
Hydroxyurea |
Teaching
Family support as they often feel guilty or responsible |
Promote wireless communication w/ NP for collab & coaching |
Disease process, complications, genetics, testing for carrier status |
Regular health maintenance visits, immunizations, PCN, coping, adequate fluids |
Avoid temp fluctuations, overexertion, & stress |
Need 24-hr access to facility that specializes in SCA |
Report & Seek Immediate Medical Attention:
- Suspected pain crisis
- Febrile illness
- Pale, listlessness, ↑ fatigue
- Unusual headache, loss of feeling, sudden weakness (stroke)
- Sudden vision changes
- Cough, SOB, chest pain (ACS)
- Limp or swollen joints
- Painful erection that won’t go down (priapism)
- Symmetric swelling of hands & feet in (dactylitis) |
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